Marouf R, Gupta R, Haider M Z, Al-Wazzan H, Adekile A D
Department of Pathology, Faculty of Medicine, Kuwait University, Safat, Kuwait.
Acta Haematol. 2003;110(1):11-5. doi: 10.1159/000072406.
While sickle cell disease (SCD) is generally mild in most Kuwaitis, because of their elevated fetal Hb levels, avascular necrosis of the femoral head (AVNFH) appears to be a common complication. It was recently documented in 26.7% of Kuwaiti children with SCD. There have, however, been no previous studies of adult patients. This is a 1-year study of consecutive, steady-state SCD patients seen in the hematology clinic of Mubarak Al-Kabeer Hospital. The patients' charts were reviewed for frequency of hospitalizations, any documented complications and steady-state complete blood count (CBC). MRI was performed using T1- and T2-weighted FATSAT sequences in coronal and axial planes with 4-mm-thick slices on a 1.5-tesla GE super-conducting magnet. Thirty-five patients were studied, consisting of 25 SS and 10 Sbeta(0)Thal patients aged between 17 and 44, with a mean age of 26.7 +/- 9.3 years. Seventeen (48.6%) had varying degrees of AVNFH; among the 70 hips examined, 29 (41.1%) were affected. Of the 17 patients affected, 11 (64.7%) were SS, while 6 (35.3%) were Sbeta(0)Thal. There were 14 (82.4%) males and 3 (17.6%) females (chi(2) = 8.6, p < 0.01). The mean age of those affected, 27.5 +/- 10.7 years, was not significantly higher than that of the unaffected (26.3 +/- 8.0 years). Eleven (64.7%) of those affected had a history of frequent vaso-occlusive crisis. No significant differences could be demonstrated in the mean CBC and Hb F values of the two groups; coexistent alpha-thal trait was not a factor in the SS group. Male gender was the only significant predisposing factor identified. While more patients with frequent vaso-occlusive crises were affected, the difference was not significant. AVNFH is, indeed, quite common among Kuwaiti SCD patients and there is a need for early institution of preventive and therapeutic protocols.
虽然镰状细胞病(SCD)在大多数科威特人中通常症状较轻,这是因为他们的胎儿血红蛋白水平较高,但股骨头缺血性坏死(AVNFH)似乎是一种常见的并发症。最近有文献记载,26.7%的科威特SCD儿童患有该病。然而,此前尚无关于成年患者的研究。这是一项对在穆巴拉克·卡比尔医院血液科就诊的连续稳态SCD患者进行的为期1年的研究。查阅了患者病历,了解住院频率、任何已记录的并发症以及稳态全血细胞计数(CBC)。使用1.5特斯拉通用超导磁体,在冠状面和轴面上采用T1加权和T2加权脂肪抑制序列(FATSAT)进行MRI检查,切片厚度为4毫米。共研究了35例患者,包括25例SS型和10例Sβ0地中海贫血型患者,年龄在17至44岁之间,平均年龄为26.7±9.3岁。17例(48.6%)有不同程度的AVNFH;在检查的70个髋关节中,29个(41.1%)受到影响。在17例受影响的患者中,11例(64.7%)为SS型,6例(35.3%)为Sβ0地中海贫血型。男性有14例(82.4%),女性有3例(17.6%)(χ2 = 8.6,p < 0.01)。受影响患者的平均年龄为27.5±10.7岁,与未受影响患者(26.3±8.0岁)相比无显著差异。11例(6